Medical Therapy for Pulmonary Arterial Hypertension

David B. Badesch, MD, FCCP; Steven H. Abman, MD; Gerald Simonneau, MD; Lewis J. Rubin, MD, FCCP; Vallerie V. McLaughlin, MD, FCCP

Disclosures

CHEST. 2007;131(6):1917-1928. 

In This Article

Abstract and Introduction

Abstract

A consensus panel convened by the American College of Chest Physicians developed guidelines for the treatment of pulmonary arterial hypertension (PAH) that were published in 2004. Subsequently, several important clinical trials have been published, and new treatments have received regulatory approval. In addition, add-on and combination therapy are being explored, which promise to open new therapeutic avenues. This article, taking into consideration studies published prior to September 1, 2006, provides an update to the previously published guidelines. The original guidelines have been summarized, a discussion of new studies has been added, and the treatment algorithm has been revised to take into account recent developments in therapy. This update provides evidence-based treatment recommendations for physicians involved in the care of patients with PAH. Due to the complexity of the diagnostic evaluation required and the treatment options available, referral of patients with PAH to a specialized center continues to be strongly recommended.

Introduction

Pulmonary arterial hypertension (PAH), defined as a mean pulmonary artery pressure (PAPm) ≥ 25 mm Hg with a pulmonary capillary wedge pressure ≤ 15 mm Hg measured by cardiac catheterization, is a disorder that may occur either in the setting of a variety of underlying medical conditions or as a disease that uniquely affects the pulmonary circulation. Irrespective of its etiology, PAH is a serious and often progressive disorder that results in right ventricular dysfunction and impairment in activity tolerance, and may lead to right-heart failure and death. The pathogenesis of PAH is complex and incompletely understood, but includes both genetic and environmental factors that alter vascular structure and function.

Dramatic advances in the treatment of PAH have occurred over the past 2 decades, based in part on seminal observations on pathogenesis made in the research laboratory. Recognizing the need to educate physicians on the diagnosis and management of PAH, the American College of Chest Physicians (ACCP) convened a multidisciplinary panel of experts from 2003 to 2004 to formulate guidelines for the approach to management of this complex condition. These evidence-based guidelines, including a comprehensive overview of treatment,[1] were published as a supplement to CHEST in 2004.[1,2,3,4,5,6,7,8] The guidelines from this original publication are presented in Table 1 .

The pace of developments in the treatment for PAH has quickened, with several important clinical trials having been published over the past 2 years that have led to regulatory approval of newer drugs and experience with combinations of existing drugs. These advances are likely to impact on the way physicians should now approach the treatment of PAH. Accordingly, the ACCP impaneled a subcommittee of the original panel to develop an update to the treatment guidelines based on these recent developments. As before, these guidelines are evidence based and the subcommittee employed the same criteria for inclusion and recommendation as were used in the previous work.

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